The term short syndrome was first created to reflect several of the features of the original reported cases. Rs3pe is listed in the worlds largest and most authoritative dictionary database of abbreviations and acronyms. Remitting seronegative symmetrical synovitis with pitting edema rs3pe is a rare. Rs3pe syndrome is a rare but welldefined syndrome known to be. By continuing to use our website, you are agreeing to our use of cookies. Rs3pe always has pitting edema, negative rf, absence of bony erosions, generally has an excellent therapeutic response to a small dose of glucocorticoid, and lacks the hladrb1 genotype that is present in both early and lateonset ra. Ab1034 the treatment approach for the management of. Rs3pe syndrome can be associated with both solid tumours like gastric, pancreatic and haematological malignancies like nonhodgkins lymphoma. Myelodysplastic syndrome mds is characterized by infective hematopoiesis with possible transformation to leukemia.
Myelodysplastic syndrome precedes the onset of remitting. Rs3pe in association with dipeptidyl peptidase4 inhibitor. It may present, as a paraneoplastic manifestation especially in older people who show a poor response to steroids. Objectiveto determine the clinical characteristics of patients with pure remitting seronegative symmetrical synovitis with pitting oedema rs3pe syndrome, and to investigate its relation with polymyalgia rheumatica pmr. Though the nature of the disease still remains illusive, the outcome is excellent. This hemitransection of the cord produces a characteristic set of clinical signs and symptoms. The rs3pe syndrome remitting seronegative symmetrical synovitis with pitting edema is a manifestation of rheumatoid arthritis in the elderly with a good prognosis. Ruben queiro, mercedes alperi, jose luis riestra and javier ballina. We describe a case of rs3pe and explore the literature surrounding this arthritis. It usually presents as an acute, symmetric polysynovitis with edema of the dorsum of the hands and feet. The association of pitting oedema and arthritis of the hands is fairly rare and specific. Remitting seronegative symmetrical synovitis with pitting edema rs3pe is a rare syndrome consisting of acute symmetrical tenosynovitis of the hands and wrists associated with pain and marked pitting edema of the dorsum of the hands or the feet. Background remitting seronegative symmetrical synovitis with pitting edema rs3pe syndrome is an acute inflammatory polyartritis and palpable pitting edema of the elderly patients. Rs3pe syndrome developing during the course of probable toxic.
So it is now thought that the key features of 3p25 microdeletion syndrome are due to absence of setd5. Upper crossed syndrome is an imbalance that occurs in the soft tissues of the thoracic and cervical areas of the body. Download pdf 250k download meta ris compatible with endnote, reference manager, procite, refworks. Pd1 inhibitors have revolutionized the treatment of advanced. Remitting seronegative symmetrical synovitis with pitting edema rs3pe syndrome is a definite subset of the seronegative symmetrical polyarthritis of the aged, characterized by dramatic pitting edema of the hands, male predominance, old age and exquisite response to. It is actually a combination of two forms of dystonia, blepharospasm and oromandibular dystonia omd. Oct 31, 2003 we report two cases of paraneoplastic remitting seronegative symmetrical synovitis with pitting edema rs3pe associated with prostatic adenocarcinoma.
Is remitting seronegative symmetrical synovitis with pitting. Rs3pe is the short form of remitting seronegative symmetrical synovitis with pitting edema. Rs3pe syndrome is a rare but well defined syndrome known to be. This syndrome could be mistaken by other polyarthritis, which normally affects the elderly.
It is characterized by symmetrical distal pitting edema with synovitis of the hands and feet that is generally very responsive to lowdose glucocorticoids. Rs3pe syndrome remitting seronegative symmetrical synovitis with pitting oedema occurs mostly in elderly people over 60 years of age and is demonstrable on the dorsum of the hands. The clinical features of the disease are characterized by bilatera we use cookies to enhance your experience on our website. Pulmonary infiltrations were detected concomitantly with the development of rs3pe syndrome, and prednisolone improved both the pulmonary and extrapulmonary lesions. Meige syndrome belongs to a group of disorders known as dystonia. Deletions and duplications of the pafah1b1 and ywhae genes in 17p. One of the patients was positive for helicobacter pylori and the other had secondary bone metastases. This unfortunate postural deviation is also known as. Rs3pe is a definite syndrome, subset of polyarthritis with favourable outcome and has a good prognosis in the older patients.
Magnetic resonance imaging mri was used to describe the anatomical structures affected by inflammation in pure rs3pe syndrome. Another important characteristic of rs3pe syndrome. Sir, rs3pe is an inflammatory disorder of unknown aetiology that affects elderly persons 1. In patients whose disease follows the expected course, an intensive search for underlying occult infections or cancers is. Mar 07, 2016 there is no formal criteria for diagnosis yet. However, the patients with pure rs3pe syndrome were. In the latter, the clinical picture of rs3pe developed after surgical intervention for the primary lesion. Is remitting seronegative symmetrical synovitis with pitting oedema rs3pe syndrome associated with hlaa2. Antiinflammatory treatment with corticosteroids leads to prompt improvement. Paraneoplastic remitting seronegative symmetrical synovitis with pitting edema syndrome should be treated with lowdose prednisolone during pembrolizumab therapy. When omd is combined with blepharospasm, it may be referred to as meiges syndrome named after henri meige, the french neurologist who first described the symptoms in detail in 1910. Fordnafiberlengthmeasurement,cellsweresynchronized withtheadditionof100nm nocodazoleinculturemedia. Lowercrossed syndrome lcs is also referred to as distal or pelvic crossed syndrome. Crps represents inhomogeneous chronic pain conditions.
About europe pmc funders joining europe pmc governance roadmap outreach. Rs3pe typically involves the joints of the extremities. Polyarthritis and pitting oedema annals of the rheumatic. It dramatically responds to low doses of glucocorticoids. The aetiology of the disease is still not well known but we can classify it as a disease with characteristic clinical features. Rs3pe can be a disease and not a syndrome has been more and more evaluated. Nivolumab induced remitting seronegative symmetrical. Case 1 was a 74yearold woman with a 30year history of type 2 diabetes who presented with edema of the hands, low grade fever, and malaise, which developed 5 weeks. In particular, deletion of pafah1b1 causes isolated lissencephaly while deletions involving both pafah1b1 and ywhae cause millerdieker syndrome. We hereby report a case of unilateral rs3pe in a patient of seronegative rheumatoid arthritis which was. Over the years, several types of tumors have been described associated with the syndrome and.
Aug 29, 2008 remitting seronegative symmetrical synovitis with pitting oedema syndrome, a rare inflammatory arthritis, commonly affects people in the older age group. Sapho syndrome genetic and rare diseases information. Rs3pe syndrome remitting symmetrical seronegative synovitis with pitting edema was first described by mc carthy in 1985. A read is counted each time someone views a publication summary such as the title, abstract, and list of authors, clicks on a figure, or views or downloads the fulltext. Remitting seronegative symmetrical synovitis with pitting edema rs3pe syndrome. The differential diagnosis of rs3pe syndrome includes rheumatoid arthritis ra, complex regional pain syndrome, gout arthritis, systemic lupus erythematosus sle, systemic. Brownsequard syndrome is caused by damage to one half of the spinal cord either left or right. We included case reports and clinical trials which providing details regarding the clinical presentation, management and prognosis of patient s with newly or previously diagnosed rs3pe. Remitting seronegative symmetrical synovitis with pitting oedema 73. Rs3pe syndrome as paraneoplastic phenomenon since 1985, tumors have been descripted in association with rs3pe 1. The most common shorthand of remitting seronegative symmetrical synovitis with pitting edema is rs3pe. Here, we describe a 67yearold man with mds with refractory anemia who developed rs3pe syndrome 3 months after the diagnosis of mds.
The cause of sapho syndrome is unknown and treatment is focused on managing symptoms. Case report open access rs3pe syndrome developing during the course of probable toxic shock syndrome. What is shorthand of remitting seronegative symmetrical synovitis with pitting edema. However, the patients with pure rs3pe syndrome were characterised by shorter duration of treatment, lower cumulative corticosteroid dose and lower frequency.
Syndrome of remitting seronegative symmetrical synovitis. Due to poor ergonomics and the sedentary nature of most jobs these days, upper crossed syndrome is amongst the most common musculoskeletal imbalances of the 21st century. A new articular syndrome described as immunrelated side effect of immunotherapy. Maroteaux lamy syndrome is an autosomal recessive disease caused by deficiency of the lysosomal enzyme nacetylgalactosamine 4sulfatase arylsulfatase b which is involved in glycosaminoglycan gag degradation 1, 2. Rheumaknowledgy macrophage activation syndrome mas. Rs3pe could be in some cases a paraneoplastic syndrome. Patients diagnosed with rs3pe syndrome at the medical college of wisconsin before 1995 were telephoned and asked about their rheumatologic course since initial diagnosis of rs3pe. Remitting seronegative symmetrical synovitis with pitting oedema rs3pe is a rare rheumatologic condition frequently missed but easily treated. It is classically described as symmetrical involvement of both upper extremities. Mergers offer an example of such changes, when subgroups merger partners merge into a common, superordinate group post.
In older people it is important to distinguish this syndrome from pmr polymyalgia rheumatica in view of the duration of treatment with steroids. It was acute in onset, severe in intensity to such an extent that she was unable to make a. Is remitting seronegative symmetrical synovitis with. The case presented is unique, combining a presenting picture of rs3pe syndrome with a. Indeed, reported here, tocilizumab treatment resulted in a remarkable suppression of clinical symptoms, accompanied by a reduction in mmp3 levels as well. A case of remitting seronegative symmetrical synovitis.
Mas is a severe, potentially fatal disease characterized by severe systemic inflammation. Treatment with prednisone 15 mg daily was instituted. Remitting seronegative symmetrical synovitis with pitting edema rs3pe is a rare syndrome. This group of neurological conditions is generally characterized by involuntary muscle contractions that force the body into abnormal, sometimes painful, movements and. Although there are overlapping clinical manifestations between rs3pe, elderlyonset rheumatoid arthritis, and polymyalgia rheumatica, rs3pe has distinct characteristics. Although the pathogenesis of rs3pe remains unknown, overproduction of il6 has been demonstrated to contribute to its development 2, 3. Hemophagocytic syndrome, hemophagocytic lymphohistiocytosis hlh, histiocytic erythrophagocytosis. The study of rs3pe syndrome has been mentioned in research publications which can be found using our bioinformatics tool below. Rs3pe is a type of benign seronegative polyarthritis that affects older people and is characterised by sudden onset of oedema and swelling of the dorsum of the hands with symmetrical polysynovitis involving wrists, metacarpophalangeal, interphalangeal, tarsal and metatarsophalangeal joints, tenosynovitis of the flexors and extensors of the hands, morning stiffness with negative rheumatoid. Rs3pe is a syndrome first described in 1985 as a distinct type of seronegative rheumatoid arthritis. Increased serum concentration of il6 has been observed in patients with rs3pe 2, 3, and therefore il6 inhibition with tocilizumab might constitute a novel strategy for treatment of rs3pe. Unilateral rs3pe in a patient of seronegative rheumatoid. Rs3pe is a type of benign seronegative polyarthritis that affects older people and is characterised by sudden onset of oedema and swelling of the dorsum of the hands with.
Rs3 pe syndrome definition of rs3 pe syndrome by medical. Onset of seven of the ten cases could be pinpointed almost. T he u ni ve rs i t y o f n e bra s ka m e di c a l c e nt e r, c e nt e r f or c ont i nui ng e duc a t i on d e s i gna t e s t hi s e nduri ng m a t e ri a l f or a. Remitting seronegative symmetrical synovitis with pitting edema rs3pe is a rare but wellreported clinical entity. Remitting seronegative symmetrical synovitis with pitting edema rs3pe is an uncommon elderlyonset rheumatic disease characterized by tenosynovitis of both flexor and extensors tendons at the wrist and at the feet. It can present as an acute onset polyarthritis with associated pitting oedema of the extremities. Cells were then washed with pbs and incubated with 100 m cldu for 15 min. Remitting seronegative symmetrical synovitis with pitting edema rs3pe. Miller, mckusick, and malvaux and report their findings in the medical literature in 1972. Isolated duplications of pafah1b1 have been associated with mild developmental delay and hypotonia, while isolated duplications. The patient started treatment with rifampicin 300 mgmonth.
Remitting seronegative symmetrical synovitis with pitting oedema rs3pe syndrome. The clinical features of the disease are characterized by bilateral pitting oedema of the hands and sudden onset of polyarthritis. Remitting seronegative symmetrical synovitis with pitting edema or rs3pe is a rare elderlyonset rheumatic syndrome. Disease bioinformatics research of rs3pe syndrome has been linked to edema, synovitis, pitting edema, remitting seronegative symmetrical synovitis with, arthritis. S ir, rs3pe is an inflammatory disorder of unknown aetiology that affects elderly persons. Remitting seronegative symmetrical synovitis with pitting. Out of all patients aged over 50 n 3,347, the rate of pmr was 0. The diagram opposite shows the effects of hemitransection of the cord on the right side of the individual. Remitting seronegative symmetrical synovitis with pitting edema rs3pe syndrome was first reported by mccarty et al. Another important characteristic of rs3pe syndrome is its association with. Patients with idiopathic rs3pe showed an excellent response to low doses of corticosteroids compared to the poor response to rs3pe in association with neoplasia. Remitting seronegative symmetrical synovitis with pitting oedema rs 3 pe syndrome is a rare inflammatory syndrome affecting the elderly, characterized by. The name 3m is derived from the initials of the three researchers who first identified it.
Remitting seronegative symmetrical synovitis with pitting edema is a rare syndrome identified by symmetric polyarthritis, synovitis, acute pitting edema of the back of the hands andor feet, and a negative serum rheumatoid factor. Weakness of the deep abdominal muscles ventrally crosses with weakness of the gluteus maximus and medius. The name of this condition comes from the initials of three researchers who first identified it. Treatment of a patient with remitting seronegative. Syndrome of remitting seronegative symmetrical synovitis with. Rs3pe as abbreviation means remitting seronegative symmetrical synovitis with pitting edema. Eight elderly men and two elderly women presented with symmetrical polysynovitis of acute onset involving most of their appendicular joints and flexor digitorum tendons associated with pitting edema of the dorsum of both hands and both feet. Synovitis inflammation of the joints, acne, pustulosis thick yellow blisters containing pus often on the palms and soles, hyperostosis increase in bone substance and osteitis inflammation of the bones. Us was performed with a linear array transducer operating at 12 mhz. We have recently encountered two cases of rs3pe that developed shortly after the initiation of a dipeptidyl peptidase4 dpp4 inhibitor and resolved markedly upon cessation of it. Rs3pe syndrome, pembrolizumab, immune checkpoint inhibitor. Remitting seronegative symmetrical synovitis with pitting oedema. The search terms included remitting seronegative symmetrical synovitis with pitting edema, remitting seronegative syndrome with pitting edema and rs3pe.
To investigate whether the remitting seronegative symmetrical synovitis with pitting edema rs3pe syndrome may represent a pdf remitting seronegative symmetrical synovitis with pitting oedema syndrome, a rare inflammatory arthritis, commonly affects people in the older age group remitting seronegative symmetrical synovitis with pitting edema. In lcs, tightness of the thoracolumbar extensors on the dorsal side crosses with tightness of the iliopsoas and rectus femoris. Remitting seronegative symmetrical synovitis with pitting edema rs3pe, first described in 1985 by mccarty et al, is a rare syndrome that is a subset of the seronegative symmetrical polyarthritis of older people. If no underlying disorder can be identified idiopathic rs3pe, this entity has an excellent prognosis and responds well to treatment. Leprosy presenting as remitting seronegative symmetrical synovitis. Therefore, we started treatment with vancomycin 1 gday and clindamycin 600 mgday, after which his fever rapidly remitted. Remitting seronegative symmetrical synovitis with pitting edema or sometimes rs 3 pe is a rare syndrome identified by symmetric polyarthritis, synovitis, acute pitting edema swelling of the back of the hands andor feet, and a negative serum rheumatoid factor. Aug 29, 2008 rs3pe syndrome can be associated with both solid tumours like gastric, pancreatic and haematological malignancies like nonhodgkins lymphoma. It can also occur as a paraneoplastic syndrome and can sometimes be a harbinger of malignancy. Remitting seronegative symmetrical synovitis with pitting oedema rs3pe syndrome is a rare inflammatory arthritis, first described by mccarty et al in 1985 characterised by abrupt onset of symmetrical distal synovitis, marked pitting edema of the dorsum of the hands andor feet, absence of rheumatoid factor rf, and favorable response to corticosteroids. Dear editor, a 48yearold female presented with pain and swelling in the bilateral proximal interphalangeal pip, metacarpophalangeal mcp and wrist joints for the past 20 days.
Patients show excellent response to low dose steroids with complete and sustained remissions. Intravenous mri contrast enhancement of inflammatory synovium. Daniel mccarty and coworkers in 1985, it was felt to be a distinct form of elderly or lateonset rheumatoid arthritis lora characterized by symmetrical synovitis of the hands and ankles with pitting edema, elevated acute phase reactants, negative rheumatoid factor rf. Neurology is the official journal of the american academy of.
The exact pathophysiology of rs3pe remains unknown. Paraneoplastic remitting seronegative symmetrical synovitis. S hort stature, h yperextensibility, o cular depression deeply set eyes, r ieger anomaly, and t eething delay. Read paraneoplastic remitting seronegative symmetrical synovitis with pitting edema rs3pe syndrome. A dose study was performed of mri intravenous contrast medium enhancement of rheumatoid synovium in the knee, comparing the enhancement with gadoteridol at doses of 0. Bir olgu sunumu abst ract rs3pe remitting seronegative, symmetric synovitis with pitting edema is a benign, seronegative syndrome, with an. Rs3pe syndrome is an inflammatory disease, which affects mainly elderly men and responds rapidly to steroids. Rs3pe is a distinct syndrome entity rather than a subset of ra. Remitting seronegative symmetrical synovitis with pitting edema a subgroup of pts with rheumatoid arthritis with sudden onset, seronegativity for rheumatoid factors management aspirin, nsaids prognosis excellent. We herein report a 62yearold man with idiopathic pulmonary fibrosis who developed remitting seronegative symmetrical synovitis with pitting edema rs3pe syndrome during followup. Pdf remitting seronegative symmetrical synovitis with. Fault tolerant circuit for syndrome measurements for the 7,1,3 qec code using steane ancilla. Recognition and management of polymyalgia rheumatica and.
Short syndrome genetic and rare diseases information center. Pdf syndrome of remitting seronegative symmetrical. On the other hand, rs3pe syndrome was diagnosed in 3 men 76, 76, and 81 years old. To investigate whether the remitting seronegative symmetrical synovitis with pitting edema rs3pe syndrome may represent a paraneoplastic disorder in a significant percentage of cases. Pdf remitting seronegative symmetrical synovitis with pitting. Frequent features frequent features the most common features in children with setd5 syndrome are also found in many other genetic. Considering the global picture, the diagnosis of rs3pe syndrome was. Remitting seronegative symmetrical synovitis with pitting oedema syndrome, a rare inflammatory arthritis, commonly affects people in the older age group. It is very suggestive of a subset of the seronegative polyarthritides of the elderly, the rs3pe syndrome or remitting seronegative symmetrical synovitis with pitting edema, as initially described by mccarty in 1985. The paraneoplastic meaning of r3spe remitting seronegative simmetrical synovitis with pitting edema syndrome introduction. If no underlying disorder can be identified, this entity has an excellent prognosis and responds well to treatment. Rs3pe syndrome developing during the course of probable. Asymmetrical involvement had also been reported, but unilateral presentation is very rare. Remitting seronegative symmetrical synovitis with pitting edema.
Meige syndrome nord national organization for rare. Dystonia is a group of movement disorders that vary in their symptoms, causes, progression, and treatments. Remitting seronegative symmetrical synovitis with pitting oedema rs3pe syndrome is a very rare condition incorporating a tenosynovitis of the hands and wrists, as well as the feet, ankles and. It is also known as brueghels syndrome and oral facial dystonia. It has not been reported as an association with rs3pe or lung cancer. To investigate clinical differences in warm and cold complex regional pain syndrome crps phenotypes. Pdf to determine the clinical characteristics of patients with pure remitting. Even if the adenocarcinoma relapses with time, the symptoms of rs3pe led our patient to an early diagnosis and successful treatment and to freedom from the disease for. Rs3pe can be associated with neoplasia and various rheumatic conditions.
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